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Rabbit Anti-Mitofusin 2/Biotin Conjugated antibody (bs-2988R-Bio)
訂購熱線:400-901-9800
訂購郵箱:sales@www.chomd.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@www.chomd.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2988R-Bio
英文名稱 Rabbit Anti-Mitofusin 2/Biotin Conjugated antibody
中文名稱 生物素標記的線粒體融合蛋白Mfn2抗體
別    名 CMT2A; CMT2A2; MARF; CPRP 1; CPRP1; Fzo; HSG; hyperplasia suppressor gene; Hypertension related protein 1; MFN 2; Mfn2; MFN2_HUMAN; Mitochondrial assembly regulatory factor; Mitofusin-2; Mitofusin2; Transmembrane GTPase MFN2.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  免疫學  神經生物學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Rat, Chicken, Dog, Pig, Cow, Horse, )
產品應用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 83kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Mitofusin 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
This gene encodes a mitochondrial membrane protein that participates in mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. This protein is involved in the regulation of vascular smooth muscle cell proliferation, and it may play a role in the pathophysiology of obesity. Mutations in this gene cause Charcot-Marie-Tooth disease type 2A2, and hereditary motor and sensory neuropathy VI, which are both disorders of the peripheral nervous system. Defects in this gene have also been associated with early-onset stroke. Two transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008].

Subunit:
Forms homomultimers and heteromultimers with MFN1.

Subcellular Location:
Mitochondrion outer membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitous; expressed at low level. Highly expressed in heart and kidney.

DISEASE:
Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2) [MIM:609260]. CMT2A2 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.
Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 6 (CMT6) [MIM:601152]; also referred to as autosomal dominant hereditary motor and sensory neuropathy VI (HMSN6). CMT6 is an autosomal dominant form of axonal CMT associated with optic atrophy.

Similarity:
Belongs to the mitofusin family.

Database links:

Entrez Gene: 9927 Human

Entrez Gene: 170731 Mouse

Entrez Gene: 64476 Rat

Omim: 608507 Human

SwissProt: O95140 Human

SwissProt: Q80U63 Mouse

SwissProt: Q8R500 Rat

Unigene: 376681 Human

Unigene: 154312 Mouse

Unigene: 437499 Mouse

Unigene: 8570 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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